Sunday, June 02, 2013

April nightmare; May resolve

When last I wrote, things were looking pretty good. I had gotten a better-than-expected result from bendamustine and rituximab. I was planning on seeing expert Dr. Thomas Kipps at UC San Diego on April 25 to discuss maintenance ideas or what to do at relapse; perhaps one of the BTK inhibitor trials would be a possibility.

And, of course, the day was getting tantalizingly close when I would be able to purchase new and better health insurance from the exchanges that will come online October 1, with the new insurance effective January 1, 2014. The new insurance, I hoped, would cover treatment at a major CLL center or two (such as UC San Diego) and would cover a stem cell transplant, if needed, which my current insurance does not.

I had managed to play for time in somewhat difficult circumstances, and was content. Until one day, around the very end of March or beginning of April, I awoke with a crick in my back.

Sometimes while sleeping I can end up in a contorted position that causes back pain. When this happens, I try to be careful for a few days to lay in such a way as to help my back muscles. Usually the pain diminishes and the problem is solved.

That's what I expected to happen here. The pain persisted, though. I had a routine appointment with Dr. Droll, my Phoenix-area oncologist, on April 5. I recall trying to get comfortable in bed the night before, and making a mental note that the pain was still there. Well, I had moved a couple of heavy boxes, against my better judgment; maybe I had put new strain on the muscles.

The CBC for the appointment showed an unusual result: my platelets had dropped from the 150s to 89. This was not the first time this had happened. Such fluctuations often occurred during my BR chemo. Dr. Droll had been wondering if I had ITP, so this might have fit that pattern. As with so many anomalous blood results on so many tests over the years, it made sense to wait for the next test, to see if this was a one-time blip or the start of a pattern.

There was also a mistake on the bloodwork. I had gone to Sonora Quest with a standing order that included LDH, or lactate dehydrogenase. The evidently dyslexic phlebotomist had input "HDL" into the computer, not LDH. So I was left with a count of cholesterol, not a reading of tissue breakdown, which is a marker of whether a cancer is growing.

Now, for those who don't know, a drop in platelets along with an elevation in LDH can be a sign of Richter's Transformation. I was aware of this, and had been on the lookout for any sign of Richter's since completing BR. Richter's can be a bit hard to disagnose, but other symptoms can include abdominal discomfort, fatigue, and sudden tumor growth.

On all those counts, I felt fine. I could detect no tumor growth. My weight had not changed significantly. I had weighed 185 in December, which is pretty normal for me. I then settled into the low 190s, probably the result of letting myself go, diet-wise. I was not adverse to the occasional dessert involving Scotch and a shortbread cookie.

A repeat CBC on April 15 showed platelets back up to 156, leading me to wonder if the one-time drop had just been an anomaly. The LDH was just mildly elevated at 247 -- two points over the reference range of 112-245. This was a little unusual, but I knew I had some abdominal nodes left over from BR, and I supposed I could be starting to relapse, and I figured I would discuss this with Dr. Kipps.

Meanwhile, the pain in my back had become worse. I could no longer sleep on my right side, just the left. I went to see my primary care doctor, who ordered an X-ray. Sure enough, it showed a compressed disc in the L5, S2 region of the spine. Ugh, I thought, this is a new and unwelcome health thing to deal with. But it did, ostensibly, explain the pain, which was becoming so bad that I needed oxycodone to fall asleep.Simultaneously, though, I developed rapid weight gain. Between April 12 and 16 I gained 10 pounds. My belly began to look distended. This raised the Richter's warning flag. Every day it seemed to get bigger. On Saturday, April 22 I went to the Sedona ER. An X-ray came back showing what the ER doc took to be a great deal of ascites. This was followed by a CT scan, which confirmed, informally, what we had been suspecting. My abdomen was filled with a huge tumorous mass. The obvious guess was Richter's Transformation.

This was not good news, but I am not one to bemoan my fate. I don't dwell on "why me?" Shit happens, and so does Richter's, to upwards of 10% of CLLers. Nobody knows why, but it does seem to come if you have had the disease for a long time, and it had been almost ten years since my diagnosis. Having abdominal nodes could be a factor, and I have had them for years. It's possible that the Epstein-Barr virus could contribute, and I had mononucleosis as a child, so EBV is still kicking around in my system somewhere. Maybe BR set up the environment for it; if you read the BR studies closely, you will note that there are always reports of Richter's on follow-up.

I figured that if this was Richter's, there would be no better place to be than in Dr. Kipps' office three days later. The pain in my back was now severe and I was unable to sleep laying down. I had to sit up in a chair and rest my head on a pillow on the dining room table. The belly continued to grow. I looked like I was pregnant with twins. For those who are used to the slow pace of CLL, which can come in on little cat feet, this was a shocking experience. Richter's had arrived with all the aplomb of Godzilla tearing through Tokyo.

Preparing for San Diego proved to have its comic moments. The night before we were to leave I suddenly realized that none of my pants would fit. Marilyn had to run to Wal-Mart at 1 a.m. to buy a selection of size 44 and 46 slacks and sweat pants. I managed to find a pair of pants that could be buttoned. Exhausted, we headed off the next day for the eight-hour drive to the sea.

Ironically, we had originally planned to make a mini-vacation of our visit. We had booked a room near the beach in Encinitas, north of San Diego. I could see the waves in the distance through the window. Talk again about the best-laid plans . . . I was in no shape to walk the beach. Everything, even the simplest task, took extraordinary effort to accomplish, and the back pain was ferocious.

My visit with Dr. Kipps is a blur in my memory. He took one look at all bloated 216 pounds of me -- and it was a look of concern -- and suspected what was happening. The exact diagnosis would have to be confirmed by PET/CT scan and lymph node biopsy. It was clear to him that diagnosis and treatment could not wait. Among other things, he was worried that the tumor could be on the verge of causing some kind of kidney damage.

It was established that treatment could not be done at UCSD because of insurance issues; I would return to Arizona the next day, and Dr. Kipps would contact Dr. Droll, which he did both by phone and fax. "Therapy in the next several days appears to be imperative," he concluded in the fax.

The back pain I was experiencing, it turns out, was not the result of a compressed disc. It was the result of a tumor pressing against nerves in my spine, something I never would have guessed when, just a few weeks before, I had awoken with a crick in my back.

This concludes Part 1. So as not to leave you in too much suspense, it did indeed turn out to be Richter's Transformation to aggressive Diffuse Large B Cell Lymphoma. CLL is now the least of my worries. The plan is to achieve the best remission possible, then move on to a stem cell transplant. I have undergone two rounds of treatment (OFAR) with what appears to be a good degree of success. After the first round, my weight dropped to 175. A CT scan before the second treatment showed a massive reduction in node size.

Needless to say, my world has turned upside down. The new normal is considerably more risky and difficult than the old one. Despite all this, there is reason for hope. People do beat Richter's. In Part 2, I will share my treatment experiences, and discuss some of the challenges ahead.

17 comments:

Diane MacKinnon
said...

David: This is a nasty turn of events. Your response to treatment is encouraging. I had a Richter's transformation in June 2012 which is documented on Chaya's website.http://updates.clltopics.org/4608-notes-from-a-richter%E2%80%99s-transformation-survivorThis is a strenuous and difficult journey which you are handling with grace and grit. I wish you great success.

David,I have been following your blog since my husband was diagonsed with CLL in October 2011. Unfortunately in February of 2012 he was diagnosed with Richter's. It is very hard to understand why this happens! I hope they figure it out one day. After a long battle and some very encouraging times, my husband passed away in January. I wish you all the best in beating this! Hang in there and never give up. Kate N.

David I'm so sorry to hear this. I read this update with an impending sense of dread. I've recently joined the CLL Forum and there are a few going through treatment for Richters on there at the moment with good results. Praying for you that the treatment works well enough to get you to transplant quickly. I can't remember - do you have a match out there? How quickly can you get the insurance for it? I'm in Australia and still have trouble getting my head around the fact that you have to pay for these things over there. Sending thoughts and prayers for both you and Marilyn. Deborah in Melbourne

David, So sorry that the battle continues for you but I rest in the knowledge that you are indeed a fighter! Pleased to hear your response to treatment, a great beginning to the battle ahead. You and M will remain in my thoughts. Thank you for being willing to share with all of us your journey. All my support and love, Wendy

David, I am so sorry to hear about this. I've been following you for about a year and was hoping you'd be able to get into one of the ibrutinib trials. You are very brave and strong and I know you will continue to battle this miserable disease. Thank you for sharing your story with us so that we may cheer you on and learn from your experiences. Hugs from Texas.

Hello David, I was DX with CLL in 2002 at age 40. I did not have treatment but saw my HGB & platelets slowly dropping and my LDH steadily rising. Found a lump in my breast in August 2009 and breast grew to "Dolly Parton" size in a week or two. I also had Richter's and was treated with R-CHOP which gave me a temporary remission from both CLL & Richter's. The CLL has creeped back. Wishing you the best in your battle and letting you know that although the battle can be difficult, we can beat this son of a gun called Richter's. Sending you hugs and keeping you in my thoughts and prayers. Janet O'Brien in Cornwall, Ontario Canada

Difficult turn of events, David. Since being diagnosed myself a little over a year ago, I've been reading and worrying about Richter's syndrome. Darn it, I wish this wasn't happening to you. I've only left a couple of comments, but I've read almost your entire blog, so it feels to me like you're a friend who shares this unfortunate cll ondition. All the best!-AM

Hello David. I have been following your blog since my wife was diagnosed in April with CLL. First, I want to thank you for your courage to share your story. The ongoing fight against this insidious disease has very much taken over our lives. My wife was being seen by Karmanos cancer center and during the start of the second round of FCR, her Doctor told us she was not responding and suggested she get into a clinical trial at OSU. Right after the Doctor suggested that, my wife had a temp of 103 and was hospitalized for a week until her fever went away (bactrim response we think). We are now awaiting test results to determine if she is a candidate for the ibrunitib study. She has 17p deletion and I am concerned about a RT diagnosis. The Karmanos doctor implied this but never confirmed before we set out to get her tested at OSU but we are hopeful for a negative RT diagnosis. Please let all of us know how you are doing. God bless you and your family. Gary

I'm sorry to read about this latest development, and can but hope the treatments you face will prove effective.

I've been reading your blog for about a year, after being diagnosed will CLL in Oct. 2011 (though I guess I'd had it for 1-2years before that). As the disease was progressing quickly, I had BR (on the national health in the UK) from April to Sept. 2012, and reacted very well as far as CLL was concerned - though got a squamous carcinoma on my nose which grew so big before they removed it that the nose had to be rebuilt with a bit of forehead. I am probably OK at the moment - for the time being.

Good luck, mate - and if you have the energy. let us know how you're getting on.

ME AND MY DIARY

February 2014 in Sedona, AZ, slimmed down to 144 lbs.

My name is David Arenson and I have chronic lymphocytic leukemia. It may kill me. Then again, it may not. Life is full of surprises, although I must admit that this is not the sort of cliffhanger that I had in mind for my 50s.

Until a few years ago, like most people, I had assumed death and disease were the province of old age, not the prime of life. I was just an average person health-wise, and feeling rather fine, thank you. I passed by the occasional wheelchair-bound person or bald-headed chemotherapy patient and didn't think that sort of thing would ever apply to me. The odds were against it, after all. Then, after a blood test at age 46, I became one of those people.

And so, my life has changed. I still enjoy the same things I always have – my beautiful and wonderful soulmate, Marilyn, and music, and walks in the woods, and cheap Asian food at strip malls, and movies in which a giant reptile threatens an entire city.

But I also have a new reality that intrudes, one where mutant B lymphocytes threaten my entire body, and one which requires becoming accustomed to unfamiliar and intimidating territory. My spleen and lymph nodes are swollen and my neck sometimes looks like that of a chipmunk storing too many nuts; bothersome nodes in my left pelvic area are a constant reminder that something is wrong with my body. Over time my immunity has been degraded and I have had to rely more on antibiotics to shake infections that once gave me no pause. I have also experienced the joys of autoimmune hemolytic anemia, of which there are none, which is a scary condition in which the body destroys its own red blood cells, and which leads to fatigue.

My CLL has had more than a physical impact. It has been quite an education -- both in terms of what I have learned about my ability to cope with what once was unthinkable, and in terms of navigating the almost freakishly contradictory world of CLL management and treatment. Needless to say, only a fool treads there without getting the lay of the land; too many local doctors are simply clueless, and even the experts can disagree. I do not claim to have it all figured out, and I expect that I never will, but I am doing my best, and I hope some of my thoughts can be of use to you.

So, if sharing my journey helps you along the way, it will have been my pleasure, something green and growing in this hard, new landscape. We help each other as we can, and this is why we have a vibrant CLL community of websites, forums, and blogs (see links below). The end of the circle is the start of the circle. What goes around comes around.

BLOGGER PROFILE

Writing has been in my blood longer than CLL. I am a former newspaper reporter and editor and co-author with Marilyn of two humor-trivia books, Disco Nixon and Rambo Reagan. Marilyn and I met at the University of California at Santa Cruz and now live in the red rock country of Northern Arizona . . . CLL Diary has been featured in CR, the magazine of the American Association for Cancer Research, and in Family Practice Management, a publication of the American Academy of Family Physicians. Besides writing about CLL, I helped establish CLL Forum, one of the largest discussion groups for patients and caregivers.

CLL PROGNOSTICS & PLANNING

As we patients eventually learn, CLL is not a one-size-fits-all disease. Some cases are indolent, some progressive, some quite aggressive. Prognostic tests can give us a much better idea of what type of CLL we are dealing with. Knowledge is power, and I believe patients should have these tests and know what they mean. They do not provide a complete picture, and sometimes clinical symptoms tell a different story than one might expect from the results, but they are important tools that can help determine the when and what of treatment.

Here are the tests: IgVH mutational status, FISH, ZAP-70 (as done at a research institution such as UC San Diego, not a commercial lab), and CD38.

My tests indicate a progressing disease. I am IgVH unmutated and ZAP-70 positive, as measured at UCSD. I developed an 11q deletion per FISH in 2006, which disappeared in 2012 for some mysterious reason, giving way to a 13q deletion. I am CD38 positive now, despite having been CD38 negative for years.

Given my tender age, I will always be navigating treatment options if I want to have any hope of living a normal life span. Knowing my test results helps me plan ahead, and knowing the possible end point in my battle with CLL helps me plan what treatments make the most sense, and in what order. Like many CLLers, I am encouraged by the progress being made by new drugs such Ibrutinib and ABT-199; not to mention the news that T-cells can be supercharged to wipe out the CLL -- in much the same ferocious way that macrophages went after my red cells during hemolysis with AIHA.

The "when and what" of treatment is a subject of great debate among CLL experts as well as patients and local doctors. I tend to take a conservative approach, ever aware of the fact that overall survival in CLL depends not just on the effectiveness of your first treatment. What you do for an encore -- your ability to respond to treatment again, and then again -- may determine how long you get to stand on the stage. The late CLL expert Dr. Terry Hamblin once wrote that CLL is a war of attrition, and I am ever mindful that such wars are won, if they can be won, slowly.

Whether my decisions ultimately are proved wise will be written in these pages. I began using single-agent rituximab (Rituxan) in 2004, adding the steroid methylprednisolone in March 2007 to combat AIHA. In October 2007, after a severe AIHA relapse that left me steroid refractory, I was treated with Rituxan + cyclophosphamide, vincristine, and prednsione (R-CVP). In January 2009, when AIHA and hemolysis of red blood cells returned, I had Rituxan + cyclophosphamide and dexamethasone (R-CD). I used this a few times to control the condition, with shorter and shorter periods until AIHA relapse. Starting in February 2010 I used Arzerra (ofatumumab) and Revlimid (lenalidomide), and then for a year and a half maintained control of the disease -- and the AIHA -- with Revlimid alone. Alas, the Revlimid came at a high price in terms of blood clotting issues, and as of 2012 I was treated with bendamustine and rituximab, which gave me a CR in the marrow and blood, leaving some swollen lymph nodes behind.

2013 is turning out to be my most challenging year yet, with the arrival of Richter's Transformation in April. Up to 10% of CLL patients can expect to develop Richter's, in which some of the CLL clones mutate into a more dangerous B cell lymphoma. Richter's is fatal in some 50% of cases, but it also can be beaten with chemotherapy and stem cell transplant. Read my latest posts for updates on my experience.

My best advice to patients is to gather all the facts you can about your CLL and then think ahead and plan ahead. Develop a long-term strategy, but expect to have to roll with the punches. And don't be rushed by doctors, family, or anyone else into a decision you are not comfortable with: Treating CLL is almost never an emergency. Take the time to learn and reflect, and then go with your intuition.

There are no guarantees that your choices will work out, of course, but at least you can rest assured that you put your heart and soul into making them. That sort of effort is the effort that can, with luck, beat cancer.

It's a peace sign, or a V for victory, not sure which

Quotes I Like

"The thing in life is not to know all the answers but rather to ask the right questions." -- Anonymous

"Hope is not the conviction that something will turn out well, but the certainty that something makes sense, regardless of how it turns out." -- Vaclav Havel

"The man who never alters his opinion is like standing water, and breeds reptiles of the mind." -- Blake

"We must be willing to let go of the life we have planned so as to have the life that is waiting for us." -- E.M. Forster

"Think of all the beauty still left around you and be happy." -- Anne Frank

“Panic is a projection that is not real. We are not just our fears. Our fears do not necessarily determine our future. This is significant.” -- Greg Anderson, lung cancer survivor

"I had a choice to make when they said I was going to die. I could chose to live the rest of my life dying, or I could chose to live life until I die. And I chose to live life'. -- Anonymous cancer patient

"Life can only be understood backwards; but it must be lived forwards." -- Soren Kierkegaard

A DISCLAIMER

I am not a doctor and I do not play one on the internet. If you take something I say as medical advice and die as a result, perhaps in your next life you will not believe everything you read on the internet.

Copyright 2005-2014 by David Arenson. All rights reserved. Material is for the personal use of CLL patients and caregivers and may not be used or reproduced for commercial purposes.